Name: Dana Beale

Theme: Porphyrias

OPTION LIST

A	5-aminolevulinic acid	I	Uroporphyrinogen III
B	Activated porphyrins and oxygen free radicals	J
C	Acute intermittent porphyria	K
D	ALA dehydratase deficiency	L
E	ALA synthase	M
F	Porphobilinogen deaminase	N
G	Porphyria cutanea tarda	O
H	Toxic porphyria	P

For each scenario below, choose the most appropriate answer from the list above. Each option may be used once, more than once or not at all.

1. Autosomal dominantly inherited porphyria with neurovisceral manifestations only, resulting from porphobilinogen deaminase deficiency.

2. Neurotoxic product(s) of heme breakdown producing neurovisceral damage in certain porphyrias.

3. Autosomal dominantly inherited (or spontaneous mutation) porphyria with cutaneous manifestations only, resulting from uroporphyrinogen decarboxylase deficiency.

4. Enzyme that catalyses the rate-limiting step of heme breakdown.

5. Product(s) of heme breakdown resulting in photosensitivity (i.e. cutaneous) damage in certain porphyrias.

ANSWERS

1. C

2. A

3. G

4. E

5. B